Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute

Background: Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP antibodies can be detected in the affected infant for the first few months of life. Common clinical manifestations of NLE include cardiac disease, notably congenital heart block and cutaneous lupus lesions. The other features of NLE including hematologic abnormalities, hepatobiliary disease, and, rarely, CNS involvement are usually underestimated. We reviewed our experience with NLE and examined the relationship between the serology of the mothers and disease in their infants. Methods: We reviewed the records in 12 cases of NLE seen at Mackay Memorial Hospital from 1984 through 2003. Results: The female to male ratio was 1.4:1. Five of the 12 patients had congenital heart block, and 7 had cutaneous lesions. Other noncutaneous manifestations included thrombocytopenia in 2, anemia in 2, and elevated aminotransferases in 3, 1 of whom also had cholestasis without evidence of other metabolic, infectious or inherited causes. One infant had a right-sided focal seizure. Anti-SSA/Ro antibodies were detected in all 12 patients, a positive ANA in 10, and anti-SSB/La antibodies in 5. The last were among the 7 who had cutaneous NLE. Anti-SSB/La antibodies were not present in any of the infants with congenital heart block. Conclusions: In pregnant women with these antibodies, fetal echocardiographic screening is useful to look for atrioventricular heart block. Asymptomatic infants borne to these mothers should be followed carefully during the first 6 months of life, as babies with NLE may initially be asymptomatic. Pediatricians caring for such children should familiarize themselves with the typical features of the disease so that they can recognize it promptly if it occurs.